Gastric dysmotility and low serum vitamin D levels in patients with gastroparesis.

Nutritional abnormalities are common in patients with gastroparesis (Gp), a disorder that may affect gastric motility and may delay emptying. The aim of this work was to identify relationships between serum nutrition markers including 25-OH vitamin D and gastric motility measures in Gp patients. We enrolled 59 consecutive gastric motility clinic patients (48 females, 11 males; mean age 44 years; 42 idiopathic; 17 diabetes mellitus) with Gp symptoms. The 25-OH vitamin D levels, for most patients slightly above the lower limit of normal (96.98 nmol/l ± 60.99), were lowest in diabetic range (DM) (75.68 nmol/l ± 34.22) vs. idiopathic (ID) (105.03 nmol/l ± 67.08) gastroparesis patients. First hour GET: one unit increase in 25-OH vitamin D level was associated 0.11% improvement (95% CI -0.22, 0.01 p=0.056) in gastric motility in all patients; this association, although marked in ID Gp patients, (-0.13, CI -0.25, -0.01 p=0.034), was not seen in DM Gp, (0.2, CI -0.45, 0.87, p=0.525). Fourth hour GET: Every unit increase of 25-OH vitamin D was associated with significant improvement in all patients, ( 0.11% CI -0.23, 0.01, p=0.053), and some weak improvement in ID group, (0.11% -0.24, 0.01, p=0.076) and absent in patients with DM (0.03, CI -0.66, 0.72, p=0.932). It is concluded that 25-OH vitamin D levels may influence gastric emptying. Underlying mechanisms for this observation might include the impact of 25-OH vitamin D on the health of the enteric nervous system. 25-OH vitamin D contributions to enteric nerve functions should be explored, particularly where autonomic nervous system comorbidities exist.

Giant biliary cystadenoma.

Biliary cystadenoma (BCA) is a rare cystic tumor which originates from intrahepatic or extrahepatic biliary ducts. Intrahepatic BCAs are more common, demonstrate a female predominance, and generally asymptomatic. Radiographic evaluation assists in the preoperative differentiation of these tumors from other cystic liver masses while resection remains the only means for definitive diagnosis. We report a case of massive mucinous intrahepatic BCA initially diagnosed as a nonresectable malignancy presenting in a female patient with progressively increasing abdominal pain and girth. Enucleation of the mass with non-anatomic resection was employed for surgical management of this intrahepatic BCA.

Postinfectious gastroparesis related to autonomic failure: a case report.

BACKGROUND AND AIM: Severe dysautonomia may be secondary to viral infections, resulting in impaired autoimmune, cardiovascular, urinary and digestive dysfunction. Herein, we present a case of a 31-year-old white female patient who had severe gastroparesis related to autonomic failure following an episode of acute gastroenteritis. This seems to be the first report providing thorough assessment of the enteric and autonomic nervous system by analysis of full-thickness small intestinal biopsies, cardiovagal testing and autopsy. HOSPITAL COURSE: This patient affected by a severe gastroparesis was treated with antiemetics, prokinetics, analgesics and gastric electrical stimulation to control symptoms. Nutritional support was made using jejunal feeding tube and, in the final stage of disease, with total parenteral nutrition. Autonomic studies revealed minimal heart rate variability and a disordered Valsalva manoeuvre although the enteric nervous system and the smooth muscle layer showed a normal appearance. Hospital courses were complicated by episodes of bacteraemia and fungemia. Serum antiphospholipid antibodies were noted but despite anticoagulation, she developed a pulmonary embolism and shortly thereafter the patient died. Autopsy revealed acute haemorrhagic Candida pneumonia with left main pulmonary artery thrombus. Sympathetic chain analysis revealed decreased myelinated axons with vacuolar degeneration and patchy inflammation consistent with Guillain-Barre syndrome. The evaluation of the enteric nervous system in the stomach and small bowel revealed no evidence of enteric neuropathy or myopathy. CONCLUSION: A Guillain-Barre-like disease with gastroparesis following acute gastroenteritis is supported by physiological and autonomic studies with histological findings.

Pulmonary thrombotic arteriopathy in patients with sickle cell disease.

BACKGROUND: Shortened life expectancy due to pulmonary hypertension (PH) is seen in 5% to 10% of patients with sickle cell disease. The principal factors suspected of causing PH are pulmonary thromboemboli (PE) and in situ arterial thrombosis. OBJECTIVE: To investigate the possible role that PE or in situ arterial thrombosis play in the development of PH in sickle cell disease. METHODS: Autopsies of 12 patients with sickle cell disease were correlated with clinical data from medical records. RESULTS: Right ventricular hypertrophy was present in 9 of 12 patients. Six patients with right ventricular hypertrophy had thrombi in large elastic pulmonary arteries. All patients with elastic artery thrombi had fresh or organized thrombi in small muscular pulmonary arteries. Hypertensive small arterial changes were present in 5 of these 6 patients. Six patients showed no thrombi in elastic arteries. Among these 6 patients, 3 had right ventricular hypertrophy and recent and organized thrombi, as well as hypertensive changes in small arteries. One of these 3 patients demonstrated plexiform-like lesions and fibrinoid necrosis of small arteries. Three patients without right ventricular hypertrophy had pneumonia or pulmonary edema with no identifiable pulmonary artery pathology. CONCLUSIONS: Arterial thrombosis with PH and cor pulmonale was regarded as the cause of death among most of these patients. Elastic artery thrombi are pulmonary thromboemboli, but pulmonary thromboemboli are always associated with widespread thrombosis of small arteries. Widespread thrombosis of small arteries alone was associated with PH in some cases. This finding suggests that pulmonary thromboemboli may be a late complication of PH and cor pulmonale and that an in situ thrombotic arteriopathy underlies the development of PH in most patients with sickle cell disease.

Solitary rectal ulcer: a rare cause of gastrointestinal bleeding in an adolescent with hemophilia A.

Solitary rectal ulcer syndrome (SRUS) is a rarely reported condition in children. The typical presentation is one of anorectal pain with passage of blood and mucus per rectum in the setting of defecation abnormalities. Diagnosis is made via endoscopy and biopsy. Solitary rectal ulcer syndrome alone is usually a benign condition; however, significant morbidity can occur if complicated by underlying disease states. We report an adolescent with hemophilia A and SRUS who presented with a rectal bleed that required blood transfusion.

Retroperitoneal mucinous cystadenoma.

Primary retroperitoneal mucinous cystadenoma is an uncommon tumor found exclusively in women. Herein, we describe a patient who had resection of a large retroperitoneal cystic mass. Histologic, immunohistochemical, and electron microscopic examination of the lining epithelial cells showed features of mesothelial cells in addition to ovarian mucinous cystadenoma. These findings suggest that these tumors arise from inclusions of mesothelial cells and subsequent mucinous metaplasia of the lining cells to form a cystadenoma. Estrogen receptors may be implicated in tumor promotion, explaining the occurrence exclusively in women.

Colonic chicken skin mucosa: association with juvenile polyps in children.

OBJECTIVES: Chicken skin mucosa is a newly described endoscopic finding associated with colonic neoplasms in adults. Chicken skin mucosa was sought in children with juvenile polyps to determine the prevalence, endoscopic features, and location. An alternative theory is proposed for the pathogenic mechanism of this finding. METHODS: Children having colonoscopy and polypectomy were prospectively evaluated for the presence of chicken skin mucosa. The location of the polyps was determined at colonoscopy; the size of removed polyps was measured during processing of samples in pathology. Biopsies from colonic chicken skin mucosa were stained with hematoxylin and eosin and mucicarmine. RESULTS: Over a 1-yr period, 27 juvenile polyps were removed from 15 children at colonoscopy. Eleven of 15 children (73%) were found to have polyps with chicken skin mucosa; overall, 43% of the polyps had associated chicken skin mucosa. Chicken skin mucosa-positive polyps were larger than chicken skin mucosa-negative polyps and were only found in the rectosigmoid colon. Lipid-laden macrophages were found in all samples of chicken skin mucosa tested. CONCLUSIONS: Chicken skin mucosa is a common finding in children with juvenile polyps. It probably is the result of local mucosal trauma, rather than a preneoplastic lesion.

Yolk sac tumor of the temporal bone: report of a case.

Yolk sac tumor (endodermal sinus tumor) is rarely encountered in the temporal bone. Facial nerve paralysis can be a primary manifestation of this condition. Histologically, the tumor can be difficult to diagnose, although elevated levels of alpha fetoprotein can facilitate its identification. In this report, we describe the case of an 18-month-old girl who developed peripheral VIIth nerve palsy and a polypoid mass in the left external ear canal 3 months following myringotomy. Computed tomography and magnetic resonance imaging revealed that the tumor involved the left external ear canal, middle ear space, and mastoid air cells. Biopsies were consistent with a yolk sac tumor. Special staining demonstrated that only a very few tumor cells were positive for alpha fetoprotein, despite the markedly elevated level of alpha fetoprotein in her serum. The patient was treated with chemotherapy, which included cisplatin, etoposide, and bleomycin. Within a period of weeks, she experienced a complete reversal of her left VIIth nerve palsy, a marked decrease in her serum alpha fetoprotein levels, and a dramatic resolution of the tumor as demonstrated radiographically. Such a successful chemotherapeutic response in this case argues against surgical intervention in other cases, particularly in view of the risk of serious complications with surgery.

Protective effects of zinc in indomethacin-induced gastric mucosal injury: evidence for a dual mechanism involving lipid peroxidation and nitric oxide.

BACKGROUND: Indomethacin causes gastric mucosal injury, although the pathogenesis is not fully understood. Zinc, is known to have gastroprotective effects in both humans and experimental animals. AIM: To determine (i) the protective effects of zinc in indomethacin-induced gastric mucosal injury in rats, and (ii) whether these cytoprotective effects are mediated by changes in gastric lipid peroxidation and/or nitric oxide synthase activity. METHODS: Gastric lesions were induced in rats by the intragastric administration of indomethacin. Morphological changes, lipid peroxidation (malondialdehyde levels) and nitric oxide synthase activity were determined in animals pre-treated with zinc sulphate and in controls. RESULTS: Indomethacin significantly increased malondialdehyde levels and decreased NOS activity. These effects were attenuated by pre-treatment with zinc (P < 0.005 and 0.0001, respectively). The protective effects of zinc were readily abolished in animals pre-treated with N-nitro-L-arginine methyl ester (L-NAME). Morphologically, indomethacin induced large areas of mucosal ulcerations, which were completely prevented by zinc pre-treatment. CONCLUSIONS: Zinc provides protection against indomethacin-induced gastric mucosal injury. These protective effects result from the inhibition of lipid peroxidation and the preservation of mucosal nitric oxide synthase.

Follicular carcinoma arising in ectopic thyroid tissue: case report with fine-needle aspiration findings.

A 32-yr-old woman was found to have a 1 x 2 cm mass in the soft tissue of the neck 1 cm lateral to the left lobe of the thyroid gland. A fine-needle aspiration biopsy showed a follicular neoplasm. The excised mass showed a follicular carcinoma arising in lateral ectopic thyroid tissue. Subsequent excision of the thyroid gland and pathological examination showed no primary carcinoma in the gland. This report illustrates a case of primary follicular carcinoma arising in a lateral ectopic thyroid tissue in the neck.

K-ras mutation in a tubular adenoma originating at an ileostomy in a familial adenomatous polyposis patient.

Genetic alterations in a tubular adenoma with severe dysplasia arising in a Brooke ileostomy of a familial adenomatous polyposis patient were analyzed. Clinical and morphological characteristics suggest that ileal mucosa progressed to colonic metaplasia and then to dysplastic adenoma. Such changes at ileostomy sites are rare, and little is known about the associated genetic alterations. To determine whether metaplastic epithelium progression to adenoma in the ileum is subject to the same mutations identified in colon carcinogenesis, we evaluated somatic genetic alterations associated with sporadic colorectal cancer development. Sequences examined included mutation cluster regions of the p53 tumor suppressor gene and the k-ras oncogene. Using polymerase chain reaction and DNA sequencing, we identified a point mutation at codon 12 of the K-ras oncogene. To our knowledge, this is the first report of a ras mutation occurring in a tumor originating from ileal mucosa.

Familial juvenile polyposis: patterns of recurrence and implications for surgical management.

BACKGROUND: Familial juvenile polyposis predisposes to the development of carcinoma of the colon. Optimum surgical management and recommended surveillance of affected individuals are still being defined. STUDY DESIGN: A retrospective review of experience with a kindred identified in 1988 was carried out. RESULTS: Of 34 living members, 15 have been investigated, and histologically typical juvenile polyps were found in 11. In each instance, polyps were most numerous in the right colon, with few polyps in the descending colon and none in the rectum. Eight patients have had subtotal colectomies with ileorectal anastomoses; the remaining patients were managed by polypectomy (with one recurrence after ten years). In addition to juvenile polyps, polyps with adenomatous or villous elements were identified in three patients. One of these patients had invasive adenocarcinoma in a large mixed polyp of the cecum. Two patients with polyps had coexisting carcinoma of the stomach. All patients have been followed up with periodic upper and lower gastrointestinal endoscopy. Polyps have recurred in the rectal remnants of three patients at a mean of 36 months after subtotal colectomy. Two patients have undergone conversion to total proctocolectomy with ileoanal anastomosis and J pouch; one patient was found to have juvenile polyps in the pouch 40 months after surgery. CONCLUSIONS: Despite the preponderance of right-sided polyps at initial diagnosis, the rapid recurrence of polyps after subtotal colectomy argues in favor of performing proctocolectomy with preservation of anal sphincter function (restorative proctocolectomy) at the time of initial surgery. Patients with a small number of polyps may choose instead to undergo periodic colonoscopy with colonoscopic polypectomy. An algorithm for surveillance and follow-up is proposed.

Does level of ligation influence results in a murine biliary obstruction model?

BACKGROUND: Despite advances in perioperative management, patients with extrahepatic biliary obstruction still experience a high rate of complications and death after surgery. The rat is commonly used as an experimental animal for research in obstructive jaundice. Ligation of the rat bile duct high in the liver hilum is assumed to produce a more severe model of biliary obstruction than low ligation. The differences are attributed to the ability of the rat bile duct to dilate. Differences in level of ligation may, thus, explain some discrepancies between studies. MATERIALS AND METHODS: To test this hypothesis, female Lewis rats underwent high ligation (HL), low ligation (LL), and sham celiotomy. Colloidal carbon clearance, bilirubin, total serum bile acids, and hematocrit were measured 12 days later. Liver and spleen weight, presence or absence of ascites, infection, and adequacy of ligation were noted and the liver was processed for routine histology and electron microscopy. RESULTS: Although bilirubin levels were higher after HL than after LL, liver and spleen weight, total serum bile salts, and phagocytic constants K and alpha were not different between these two groups. Gross, histologic, and ultrastructural appearance did not differ between HL and LL groups. CONCLUSION: High ligation causes greater hyperbilirubinemia than low ligation, but does not alter other parameters including phagocytic constants. The present study does not confirm the hypothesis that HL creates a more severe model than LL; therefore, it is unlikely that differences in level of ligation explain variability in results between studies.

Experimental autoimmune hepatitis in mice following immunization with syngeneic liver proteins.

In this study, we describe the successful induction of experimental autoimmune hepatitis by immunizing inbred strains of mice with syngeneic liver antigens. Hepatic lesions, presumably based on autoimmune mechanisms, induced after 3 weekly immunizations were of only moderate degree. Of the various mouse strains used, C57BL/6 mice showed the most prominent histological changes of the liver, which were accompanied by elevated serum alanine aminotransferase levels. This system offers a potential model for further investigating experimental autoimmune hepatitis.

Localization of small intestinal bleeding. The role of intraoperative endoscopy.

The preoperative identification of a bleeding site is not always possible, particularly when bleeding originates in the small intestine. Small vascular abnormalities, such as the telangiectatic lesion described in this report, comprise about 40-60% of such cases. Preoperative location using arteriography, radionuclide bleeding scan, and enteroclysis were nondiagnostic. The lesion was demonstrated by intraoperative endoscopy. A segment of small intestine was resected, and the patient made an uneventful recovery.

Diagnosis and natural history of extramammary tumors metastatic to the breast.

BACKGROUND: In patients with known extramammary malignancies, metastatic disease should be considered in the differential diagnosis of a breast mass. STUDY DESIGN: Retrospective review. RESULTS: From January 1, 1980 to December 31, 1992, nine women (ages 25 to 67 years) were identified with breast masses, the biopsies of which proved to be metastatic from other sites. All patients presented with palpable breast masses. Mammograms were obtained in five patients; all demonstrated the palpable abnormality. Two of three mammograms showing multiple nodules were evaluated as suggestive of benign disease. In three patients, breast metastases were the presenting symptom of an occult primary tumor. The remaining six patients were diagnosed with metastatic disease in the breast from ten months to 15 years after the initial diagnosis (mean of 5.5 years) of an extramammary malignancy. One-half of the patients presented five or more years after the initial diagnosis. Breast metastases were associated with disseminated metastatic disease in eight of the nine patients. Six patients died after a mean interval of 8.2 months (range of 3.5 to 35 months) from diagnosis of breast metastases. One patient was unavailable for follow-up evaluation and is presumed dead. CONCLUSIONS: Metastatic disease should be considered in the differential diagnosis of a palpable breast mass, particularly if there is a history of extramammary malignancy. The presence of multiple or bilateral well-circumscribed nodules may suggest a benign process on mammography. Breast metastasis is usually indicative of diffuse metastatic disease and a poor prognosis. Biopsy and careful review of previous pathologic material assures prompt treatment and avoids an unnecessary radical operation.